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19 Cards in this Set
- Front
- Back
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why is excess NH3 thought to be toxic because the TCA cycle intermediate aKG is converted to Glu?
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glutamate DH is reversible!
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Most of the NH3 generated by aa ctabolis comes from the coupling of a transamination reaction with the _______ ______ reaction. The netresult of the two reactions is an _______ _______ of the transaminated aa
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glutamate DH
oxidative deamination |
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____ and ____ can be nonoxidatively deaminated by a dehydratase reaction NH3 is also generated by the nonoxidative deamidations of ___ and ___
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ser thr
gln and asn |
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what are the two sources of nitrogens for urea
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NH3 and alpha amino group of Asp
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how many ATP ares spent in urea synthesis
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4
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what is the committed step in urea synthesis
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synthesis of carbamoylP from NH3 via CPSI
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what is the initial step in phe catabolsim
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hydroxylation to tyrosine
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what is low in PKU
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phe hydroxyse activity
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What do you give infants with PKU
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low phe and ample tyr
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how do you catabolize branched chain aa
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transamination and then oxidative decarboxylation of the alpha keto acid. The next steps look like beta oxidation of FA
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what four aa can form propionyl Coa
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thr met ile val
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what is one thing that builds up if vit B12 deficient
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methylmalonate accumulation which leads to neuro damage
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how do you get from glu to gln
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glutaminase
glutamine synthetase |
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what activates CP synthetase
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N acetylglutamate
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what is deficient in MSUD
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Branched chain alpha keto acid DH
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aKG + asp -->
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glu + OAA
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ser + serine dehydrogetase -->
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pyruvate + NH3
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Asn --> asp by what enzymes
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asparginase
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what builds up if you have thiamine deficiency? biotin def? B12 def?
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aKB
propionyl COA methylmalonylCoa |
